Prophylactic implantation of cardioverter-defibrillator in patients with advanced light-chain amyloidosis-A pilot

Hui-Qiang Wei1, Jinghua Wang2, Yuanhong Liang1

  • 1Department of Cardiology, Guangdong Cardiovascular Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences) Southern Medical University Guangzhou China.

Journal of Arrhythmia
|April 21, 2025
PubMed

Insights

Implantable cardioverter-defibrillators (ICDs) effectively treated ventricular arrhythmias (VAs) in advanced light chain (AL) amyloidosis patients. Prophylactic ICDs may improve survival in selected AL amyloidosis patients with wider QRS duration and bundle branch block.

Area of Science:

  • Cardiology
  • Electrophysiology
  • Cardiac Amyloidosis

Background:

  • Ventricular arrhythmias (VAs) and electromechanical dissociation are leading causes of sudden cardiac death (SCD) in light chain (AL) amyloidosis.
  • Implantable cardioverter-defibrillators (ICDs) are rarely used in advanced AL amyloidosis due to poor prognosis.

Purpose of the Study:

  • To evaluate the efficacy and safety of prophylactic ICD implantation in patients with advanced cardiac AL amyloidosis.
  • To determine the prevalence of VAs and appropriate ICD therapies in this patient population.

Main Methods:

  • Prospective recruitment of 10 patients with advanced cardiac AL amyloidosis receiving prophylactic ICDs.
  • Primary endpoint: prevalence of VAs and appropriate ICD therapies.
  • Secondary endpoint: all-cause mortality during follow-up.

Main Results:

  • Sustained ventricular tachycardia/ventricular fibrillation (VT/VF) occurred in 40% of patients during a mean 12.1-month follow-up.
  • 3 patients received appropriate ICD therapies (ATP or shock); no inappropriate shocks were recorded.
  • Patients with VT/VF had significantly wider QRS duration and higher incidence of bundle branch block (BBB)/interventricular conduction delay (IVCD).

Conclusions:

  • VAs are common in advanced AL amyloidosis, and ICD therapy is effective for sustained VA treatment.
  • Prophylactic ICD implantation may be beneficial for selected advanced AL amyloidosis patients, particularly those with wider QRS duration and BBB/IVCD.
  • Preliminary data suggest prophylactic ICDs could improve survival rates in this high-risk group.
Abstract

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